Pulmonary cystic destruction in systemic sclerosis and antisynthetase syndrome associated non-specific interstitial pneumonitis: a case series of a novel radiological phenotype.

Abstract

Objectives: To describe a novel radiographic phenotype of systemic autoimmune rheumatic disease-associated interstitial lung disease (SARD-ILD) observed within a regional specialist multidisciplinary (MDT) service.

Methods: This was a retrospective case series of patients with SARD-ILD complicated by radiographic pulmonary cystic destruction. Cases were identified through review of MDT records from the North Bristol SARD-ILD service. Cases with MDT reported 'cystic changes' were identified and clinico-radio-pathological features reviewed.

Results: Five cases of SSc-associated ILD (SSc-ILD) and two cases of antisynthetase syndrome-associated ILD (ASyS-ILD) with cystic changes were identified among a total of 108 SARD-ILD patients. All seven cases presented with radiological patterns of cellular non-specific interstitial pneumonia (NSIP). The median age at diagnosis with SARD was 33 years and six cases had ILD identified within 6 months of SARD diagnosis. The cohort was ethnically diverse, with one ex-smoker. Microcystic destructive changes appeared and progressed within areas of ground glass despite standard-of-care immunomodulation. Changes are radiographically and histologically distinct from traction bronchiolectasis, honeycombing and smoking-related lung disease. Histological specimens were available for two cases confirming fibrotic NSIP, with one including affected tissue demonstrating intimal thickening of the pulmonary vasculature. Over a median follow-up of 49 months, all cases remain alive and transplant free; five fulfilled criteria for progressive disease and six required ambulatory oxygen.

Conclusions: This represents the first western cohort describing microcystic destructive pulmonary changes in SSc-ILD and the first report in ASyS-ILD. Systematic case identification is required to determine demographic associations and prognostic implications.